Hermansky-Pudlak Syndrome with Interstitial Pneumonia without Mutation of HSP1 Gene
スポンサーリンク
概要
- 論文の詳細を見る
A 57-year-old man with occulocutaneous albinism was admitted to our hospital because of exertional dyspnea and an abnormal shadow on chest roentgenogram. Chest CT revealed diffuse interstitial shadows with reticulonodular opacities in the bilateral whole lung fields and his pulmonary function test was consistent with a restrictive finding. Histologically, intraluminal diffuse fibrosis and interstitial fibrosis existed and ceroid-like materials within alveolar macrophages were demonstrated in a transbronchial lung biopsy specimen. In addition, because platelet dysfunction and ceroid-like materials within the reticuloendothelial cells of urine and bone marrow aspiration were recognized, we made a diagnosis of Hermansky-Pudlak syndrome (HPS). Gene analysis of the patient’s peripheral blood cells did not reveal that he was a compound homogeneity for HPS1 gene mutations. Concerning treatment, although corticosteroid therapy was administered, his clinical symptoms and abnormal chest shadow have not changed.
- 社団法人 日本内科学会の論文
- 2005-06-01
著者
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二木 芳人
川崎医科大学呼吸器内科
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Matsushima Toshiharu
昭和大学 医学部臨床感染症学
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Matsushima Toshiharu
東北大学加齢医学研究所 抗感染症薬開発研究部門
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Niki Yoshihito
昭和大学 医学部臨床感染症学
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Niki Y
Showa University School Of Medicine Department Of Clinical Infectious Diseases
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Yoshihito Niki
Department Of Respiratory And Internal Medicine Kawasaki Medical School
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Niki Yoshihito
Division Of Respiratory Diseases Department Of Medicine Kawasaki Medical School
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Kobashi Yoshihiro
川崎医科大学 医学部内科学(呼吸)
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Niki Yoshihito
Showa University School Of Medicine Department Of Clinical Infectious Diseases
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Kubota Y
Division Of Respiratory Diseases Department Of Medicine Kawasaki Medical School
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Yoshida Kouichirou
昭和大学 医学部臨床感染症学
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MIYASHITA Naoyuki
Division of Respiratory Diseases, Department of Medicine, Kawasaki Medical School
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MATSUSHIMA Toshiharu
Division of Respiratory Diseases, Department of Medicine, Kawasaki Medical School
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Niki Yoshihito
Department Of Respiratory And Internal Medicine Kawasaki Medical School
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Manabe Takao
大阪市立総合医療センター
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Matsushima T
Division Of Respiratory Diseases Department Of Medicine Kurashiki Daiichi Hospital
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Kobashi Yoshihiro
Division of Respiratory Diseases, Department of Medicine, Kawasaki Medical School
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MATSUSHIMA Toshiharu
Junpukai Kurashiki Daiichi Hospital
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YOSHIDA Kouichiro
Division of Respiratory Diseases, Department of Medicine, Kawasaki Medical School
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Kobashi Yoshihiro
Division Of Respiratory Diseases Department Of Medicine Kawasaki Medical School
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Miyashita Naoyuki
Division Of Respiratory Diseases Department Of Internal Medicine
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Matsushima Toshiharu
Division Of Arespiratory Diseases Department Of Medicine Kawasaki Medical School
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Matsushima Toshiharu
The Division Of Respiratory Diseases Department Of Medicine Kawasaki Medical School
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Takashima M
Japan Collection Of Microorganisms Riken (the Institute Of Physical And Chemical Research)
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Yoshida Koichiro
Department Of Clinical Infectious Diseases School Of Medicine Showa University
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Manabe T
Osaka City General Hospital
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Miyashita Naoyuki
Department Of Respiratory Diseases Kawasaki Medical School
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Nishino Yoshinori
Japanese Society Of Chemotherapy (jsc)
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Miyashita Naoyuki
Department Of Respiratory Medicine Kawasaki Medical School
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Kobashi Yoshihiro
Division Of Respiratory Disease Department Of Medicine Kawasaki Medical School
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Kobashi Yoshihiro
The Division Of Respiratory Diseases Department Of Medicine Kawasaki Medical School Kawasaki Hospita
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Niki Yoshihito
Division Of Clinical Infectious Diseases Department Of Internal Medicine Showa University School Of Medicine
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Yoshida Kouichirou
Division Of The Respiratory Diseases The Department Of Medicine Kawasaki Medical School
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Miyashita Naoyuki
Division of Respiratory Disease, Department of Medicine, Kawasaki Medical School, Japan
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